Primary Linitis Plastica of the Colon: Report
of a Case a n d R e v i e w of the L i t e r a t u r e *
JAYANTA R.OY CHOWDHURY, M.B.B.S., M.R.C.P.
(U.K.),
KAMALA DAS, M.B.B.S., M.R.C.O.G. (LONI~.), KIRON M. DAS, M.D., Ph.D. t From the Nassau County Medical Center, East Meadow, New York
"LINITIS PLASTICA" iS a term commonly used to describe a diffusely infiltrating carcinoma of a hollow organ, which retains its shape, but becomes thick, rigid and diminished in diameterA T h e lesion was first recognized in the stomach by Lieutaud 12 in 1779, and Andral 3 reported the first case in 1829 as a case of simple hypertrophy of the stomach. Because of the similarity of the interweaving collagen tissue of the lesion to linen cloth, Brinton s named the condition "linitis plastica" (Greek meaning: linen cloth or net) in 1865. Brinton, s however, was not sure of the neoplastic nature of the disease. It remained for Howard 9 to emphasize the malignant nature of the lesion in 1933. Since the histologic appearance of the lesion is so variable, 21, it seems reasonable to base the diagnosis on its morphologic appearance which is so characteristic. T h e stomach is the most common site of the linitis plastica type of carcinoma, and accounts for 99 per cent of this kind of lesion. 22 Approximately 10 to 15 per cent of all gastric carcinomas fall in this category. 17 T h e large bowel contributes 0.9 per cent and the gallbladder and breast together, 0.1 per cent. 22 In a series of 12,000 cases of carcinoma of the colon, only 11 cases of linitis plastica were found, an incidence of less than one *Received for publication September 13, 1974. t Present address: Albert Einstein College of Medicine, Bronx, New York. Address reprint requests to Dr. Chowdhury, Nassau County Medical Center, 2201 Hempstead Turnpike, East Meadow, New York 11554.
in 1,000.7 T h e majority of the cases of linitis plastica of the colon are secondary to similar lesions of the stomach and gallbladder 9 About I00 cases of secondary linitis plastica of the colon have been reported so far. 2 Primary linitis plastica of the colon is even more rare, there being only 14 unequivocal cases in medical literature.a, 2. 11, 13, 14, lS, 19, 21, 22 I n a d d i t i o n to these, six cases 3, 6. s, 10 that the authors thought were examples of primary linitis plastica of the colon have been reported. But no definite evidence of absence of neoplastic lesions of the stomach and gallbladder is available in these reports, it is difficult to include these as primary lesions of the colon. In their report of 11 cases of linitis plastica-type carcinomas of the colon, Fahl et al. 7 did not differentiate between primary and secondary lesions. T h e purpose of the present paper is to review the features of the 14 cases reported in the literature, and in addition, to report a new case of primary iinitis plastica of the colon. R e p o r t of a C a s e A 38-year-old Negro woman was admitted with complaints of episodic abdominal pain, diarrhea alternating with constipation, and gross rectal bleeding for three weeks. She had had no bowel problem in the past, and there was no history of weight loss. Menstrual periods were regular, but during the period prior to admission she had experienced dysmenorrhea, which was unusual for her,
The past history was unremarkable, except that the patient had recurrent superficial thrombophlebids from varicose veins of the leg during the preceding six years.
332 Dis. Col. & Reet.
May-June 1975
Volume 18 9 Number 4
Volume 18 Number 4
PRIMARY LINITIS PLASTICA OF T H E C O L O N
333
FIG. 1. Barium-enema study, showing long, constricted segment of sigmoid colon.
Physical examination showed that the patient was grossly obese, with slight pallor, b u t no cyanosis, jaundice or edema. T h e r e was no lymphadenopathy. She had a deep diffuse tenderness over the left iliac fossa and the left lumbar region. Shifting dullness was present on percussion, but marked obesity precluded estimation of the exact quantity of ascitic fluid. Except for a positive test for occult blood in the stool, the rectal examination was negative. Pelvic examination revealed a nodular mass in the pouch of Douglas and fullness of both fornices. It was possible to introduce the sigmoidoscope up to 18 cm only. Up to this level the mucosa of the rectum and sigmoid colon was normal, but blood was seen coming from above this level. Varicose veins were observed in the lower extremities, but no deep venous thrombosis was present. T h e rest of the physical examination was within normal limits. T h e initial blood studies showed: hemoglobin 12 g/100 ml; hematocrit 36 per cent; leukocyte count 7,200 cu ram. neutrophils 73 per cent, lymphocytes 26 per cent, and monocytes 1 per cent. P r o t h r o m b i n time was 13.9 sec (control 11.8 sec). Blood glucose was 107 rag/100 ml; blood urea nitrogen 10 rag/100 ml; sodium 136 mEq/1, potassium 3.8 mEq./1, bicarbonate 24 mEq/1, chloride 98 mFq/1; total protein 7.4 g/100 ml, albumin 4.33 g/100 ml; calcium 9.8 rag/100 ml; inorganic phos-
phorus 2.6 mg/100 ml; creatinine I mg/100 ml: bilirubin 0.4 mg/100 ml; alkaline phosphatase 57 IU; lactic dehydrogenase 194 IU; serum glutamic oxaloacetic acid transaminase 100 IU. Urinalysis was within normal limits. Barium-enema studies showed a stricture of the proximal sigrnoid colon, 8 to 10 cm long, with minimal mucosal destruction. T h e outline of the colonic wall at the region of the stricture was gen. erally smooth, but on some views an appearance of shelving was suggested (Fig. 1). T h e radiologic picture was t h o u g h t to be consistent with an inflammatory lesion of the colon, or an extracolonic neoplastic lesion enveloping and constricting the colon. T h e rest of the colon and the terminal ileum were normal. An upper gastrointestinal series and small bowel follow-through were normal, and so was the chest x-ray. An intravenous pyelogram revealed an extrinsic pressure defect on the dome of the urinary bladder, b u t subsequent cystoscopy showed no abnormality. During the following two weeks moderately severe ascites developed. T h e ascitic fluid was grossly bloody, with a protein content of 4.6 g/100 ml. Cytologic examination of the fluid revealed malig. nant cells of adenocarcinomatous type ("Pap" class V). Cytology of the uterine cervical smear was also positive for adenocarcinoma ("Pap" class V). Uterine cervical biopsy, however, was negative for malignancy. At this stage a p r i m a r y colonic neo-
334
Dis. Col. & Reef. May-J'une 1975
CHOWDHURY, ET AL.
mass on the right side of the pelvis. A skeletal survey was unrevealing. Immediately following completion of these investigations, while the patient was being prepared for operation, she suddenly died of massive pulmonary embolism. This was approximately four weeks after her initial admission. Postmortem examination showed a massive pulmonary embolism with infarction. T h e sigmoid colon contained an area of thickened, rigid wall, 10 cm long, with a smooth glistening inner lining, devoid of normal mucosal pattern (Fig. 2). Tumor-like masses were found in both ovaries (Fig. 3). There was evidence of lymph-node metastasis in the intra-abdominal, para-aortic, and lower thoracic para-aortic lymph nodes. T h e peritoneum was seeded with numerous sites of metastasis. Microscopic examination of the colonic lesion showed a large n u m b e r of darkly stained malignant cells, with large nuclei and scanty cytoplasm. Some malignant cells contained a large amount of amorphous mucoid material, giving them a "signet ring" appearance. Other malignant cells were seen in small glandular formation (Fig. 4). A similar histologic picture was found in the ovarian metastases. T h e stomach, gallbladder, liver, and other abdominal viscera were normal both macroscopically and microscopically. FIG. 2. Autopsy specimen of the involved area of the sigmoid colon.
plasm involving the ovary or a primary ovarian neoplasm involving the colon was suspected. A repeat barium-enema study three weeks after the initial study showed the same picture as before. A B-mode sonogram showed ascites, a large solid mass on the left side of the pelvis, and a smaller
Discussion T h e c l i n i c a l d a t a o f 15 cases o f p r i m a r y linitis plastica of the colon and rectum a r e p r e s e n t e d i n T a b l e 1. A n a l y s i s o f t h e d a t a d o e s n o t s h o w a n y sex p r e p o n d e r a n c e , there being seven male and eight female patients. Ages of the patients ranged from 24 t o 72 years.
Fro. 3. Metastatic involvement of b o t h ovari~.
Volume 18 Number 4
PRIMARY LINITIS PLASTICA OF T H E COLON
335
FIG. 4. Histologic picture of the colonic lesions, showing "signet ring" type of malignant cells and malignant cells forming small acini. (OTiginaI magnification • photographic enlargement ?
of a Case a n d R e v i e w of the L i t e r a t u r e *
JAYANTA R.OY CHOWDHURY, M.B.B.S., M.R.C.P.
(U.K.),
KAMALA DAS, M.B.B.S., M.R.C.O.G. (LONI~.), KIRON M. DAS, M.D., Ph.D. t From the Nassau County Medical Center, East Meadow, New York
"LINITIS PLASTICA" iS a term commonly used to describe a diffusely infiltrating carcinoma of a hollow organ, which retains its shape, but becomes thick, rigid and diminished in diameterA T h e lesion was first recognized in the stomach by Lieutaud 12 in 1779, and Andral 3 reported the first case in 1829 as a case of simple hypertrophy of the stomach. Because of the similarity of the interweaving collagen tissue of the lesion to linen cloth, Brinton s named the condition "linitis plastica" (Greek meaning: linen cloth or net) in 1865. Brinton, s however, was not sure of the neoplastic nature of the disease. It remained for Howard 9 to emphasize the malignant nature of the lesion in 1933. Since the histologic appearance of the lesion is so variable, 21, it seems reasonable to base the diagnosis on its morphologic appearance which is so characteristic. T h e stomach is the most common site of the linitis plastica type of carcinoma, and accounts for 99 per cent of this kind of lesion. 22 Approximately 10 to 15 per cent of all gastric carcinomas fall in this category. 17 T h e large bowel contributes 0.9 per cent and the gallbladder and breast together, 0.1 per cent. 22 In a series of 12,000 cases of carcinoma of the colon, only 11 cases of linitis plastica were found, an incidence of less than one *Received for publication September 13, 1974. t Present address: Albert Einstein College of Medicine, Bronx, New York. Address reprint requests to Dr. Chowdhury, Nassau County Medical Center, 2201 Hempstead Turnpike, East Meadow, New York 11554.
in 1,000.7 T h e majority of the cases of linitis plastica of the colon are secondary to similar lesions of the stomach and gallbladder 9 About I00 cases of secondary linitis plastica of the colon have been reported so far. 2 Primary linitis plastica of the colon is even more rare, there being only 14 unequivocal cases in medical literature.a, 2. 11, 13, 14, lS, 19, 21, 22 I n a d d i t i o n to these, six cases 3, 6. s, 10 that the authors thought were examples of primary linitis plastica of the colon have been reported. But no definite evidence of absence of neoplastic lesions of the stomach and gallbladder is available in these reports, it is difficult to include these as primary lesions of the colon. In their report of 11 cases of linitis plastica-type carcinomas of the colon, Fahl et al. 7 did not differentiate between primary and secondary lesions. T h e purpose of the present paper is to review the features of the 14 cases reported in the literature, and in addition, to report a new case of primary iinitis plastica of the colon. R e p o r t of a C a s e A 38-year-old Negro woman was admitted with complaints of episodic abdominal pain, diarrhea alternating with constipation, and gross rectal bleeding for three weeks. She had had no bowel problem in the past, and there was no history of weight loss. Menstrual periods were regular, but during the period prior to admission she had experienced dysmenorrhea, which was unusual for her,
The past history was unremarkable, except that the patient had recurrent superficial thrombophlebids from varicose veins of the leg during the preceding six years.
332 Dis. Col. & Reet.
May-June 1975
Volume 18 9 Number 4
Volume 18 Number 4
PRIMARY LINITIS PLASTICA OF T H E C O L O N
333
FIG. 1. Barium-enema study, showing long, constricted segment of sigmoid colon.
Physical examination showed that the patient was grossly obese, with slight pallor, b u t no cyanosis, jaundice or edema. T h e r e was no lymphadenopathy. She had a deep diffuse tenderness over the left iliac fossa and the left lumbar region. Shifting dullness was present on percussion, but marked obesity precluded estimation of the exact quantity of ascitic fluid. Except for a positive test for occult blood in the stool, the rectal examination was negative. Pelvic examination revealed a nodular mass in the pouch of Douglas and fullness of both fornices. It was possible to introduce the sigmoidoscope up to 18 cm only. Up to this level the mucosa of the rectum and sigmoid colon was normal, but blood was seen coming from above this level. Varicose veins were observed in the lower extremities, but no deep venous thrombosis was present. T h e rest of the physical examination was within normal limits. T h e initial blood studies showed: hemoglobin 12 g/100 ml; hematocrit 36 per cent; leukocyte count 7,200 cu ram. neutrophils 73 per cent, lymphocytes 26 per cent, and monocytes 1 per cent. P r o t h r o m b i n time was 13.9 sec (control 11.8 sec). Blood glucose was 107 rag/100 ml; blood urea nitrogen 10 rag/100 ml; sodium 136 mEq/1, potassium 3.8 mEq./1, bicarbonate 24 mEq/1, chloride 98 mFq/1; total protein 7.4 g/100 ml, albumin 4.33 g/100 ml; calcium 9.8 rag/100 ml; inorganic phos-
phorus 2.6 mg/100 ml; creatinine I mg/100 ml: bilirubin 0.4 mg/100 ml; alkaline phosphatase 57 IU; lactic dehydrogenase 194 IU; serum glutamic oxaloacetic acid transaminase 100 IU. Urinalysis was within normal limits. Barium-enema studies showed a stricture of the proximal sigrnoid colon, 8 to 10 cm long, with minimal mucosal destruction. T h e outline of the colonic wall at the region of the stricture was gen. erally smooth, but on some views an appearance of shelving was suggested (Fig. 1). T h e radiologic picture was t h o u g h t to be consistent with an inflammatory lesion of the colon, or an extracolonic neoplastic lesion enveloping and constricting the colon. T h e rest of the colon and the terminal ileum were normal. An upper gastrointestinal series and small bowel follow-through were normal, and so was the chest x-ray. An intravenous pyelogram revealed an extrinsic pressure defect on the dome of the urinary bladder, b u t subsequent cystoscopy showed no abnormality. During the following two weeks moderately severe ascites developed. T h e ascitic fluid was grossly bloody, with a protein content of 4.6 g/100 ml. Cytologic examination of the fluid revealed malig. nant cells of adenocarcinomatous type ("Pap" class V). Cytology of the uterine cervical smear was also positive for adenocarcinoma ("Pap" class V). Uterine cervical biopsy, however, was negative for malignancy. At this stage a p r i m a r y colonic neo-
334
Dis. Col. & Reef. May-J'une 1975
CHOWDHURY, ET AL.
mass on the right side of the pelvis. A skeletal survey was unrevealing. Immediately following completion of these investigations, while the patient was being prepared for operation, she suddenly died of massive pulmonary embolism. This was approximately four weeks after her initial admission. Postmortem examination showed a massive pulmonary embolism with infarction. T h e sigmoid colon contained an area of thickened, rigid wall, 10 cm long, with a smooth glistening inner lining, devoid of normal mucosal pattern (Fig. 2). Tumor-like masses were found in both ovaries (Fig. 3). There was evidence of lymph-node metastasis in the intra-abdominal, para-aortic, and lower thoracic para-aortic lymph nodes. T h e peritoneum was seeded with numerous sites of metastasis. Microscopic examination of the colonic lesion showed a large n u m b e r of darkly stained malignant cells, with large nuclei and scanty cytoplasm. Some malignant cells contained a large amount of amorphous mucoid material, giving them a "signet ring" appearance. Other malignant cells were seen in small glandular formation (Fig. 4). A similar histologic picture was found in the ovarian metastases. T h e stomach, gallbladder, liver, and other abdominal viscera were normal both macroscopically and microscopically. FIG. 2. Autopsy specimen of the involved area of the sigmoid colon.
plasm involving the ovary or a primary ovarian neoplasm involving the colon was suspected. A repeat barium-enema study three weeks after the initial study showed the same picture as before. A B-mode sonogram showed ascites, a large solid mass on the left side of the pelvis, and a smaller
Discussion T h e c l i n i c a l d a t a o f 15 cases o f p r i m a r y linitis plastica of the colon and rectum a r e p r e s e n t e d i n T a b l e 1. A n a l y s i s o f t h e d a t a d o e s n o t s h o w a n y sex p r e p o n d e r a n c e , there being seven male and eight female patients. Ages of the patients ranged from 24 t o 72 years.
Fro. 3. Metastatic involvement of b o t h ovari~.
Volume 18 Number 4
PRIMARY LINITIS PLASTICA OF T H E COLON
335
FIG. 4. Histologic picture of the colonic lesions, showing "signet ring" type of malignant cells and malignant cells forming small acini. (OTiginaI magnification • photographic enlargement ?
